硫化物氧化为硫酸盐
中文名称
通路描述
虽然人体对含硫氨基酸（SAA）非常经济，但多余的 SAA 通过半胱氨酸降解为有毒的硫化氢，必须进行处理。氧化该气体的途径定位于线粒体，高度保守，指向生命曾浸泡在富含硫化物的水中。硫化物氧化途径包含五个反应，其中硫转移从硫代硫酸盐到谷胱甘肽的反应尚未完全表征清楚。已鉴定出一个与乙醛酸脑病相关的酶突变，该突变与组织硫化物升高相关（Stipanuk & Ueki 2011）。
英文描述
Sulfide oxidation to sulfate While the human body is very economical with sulfur amino acids (SAA), superfluous SAA are degraded via cysteine to toxic hydrogen sulfide which must be dealt with. The pathway to oxidize this gas is localized to mitochondria and is highly conserved, pointing back to a time when life was immersed in sulfide-rich waters.
The pathway for sulfide oxidation consists of five reactions, one of which, the sulfur transfer from thiosulfate to glutathione, is still to be characterized fully. A mutation in one enzyme has been identified that is associated with ethylmalonyl encephalopathy and where tissue sulfide is elevated (Stipanuk & Ueki 2011).
The pathway for sulfide oxidation consists of five reactions, one of which, the sulfur transfer from thiosulfate to glutathione, is still to be characterized fully. A mutation in one enzyme has been identified that is associated with ethylmalonyl encephalopathy and where tissue sulfide is elevated (Stipanuk & Ueki 2011).
所含基因
6 个基因